{"id":12536,"date":"2024-10-11T19:57:18","date_gmt":"2024-10-11T17:57:18","guid":{"rendered":"https:\/\/valintermed.com\/?p=12536"},"modified":"2024-10-11T19:57:18","modified_gmt":"2024-10-11T17:57:18","slug":"pervichnyy-biliarnyy-holangit","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/","title":{"rendered":"Primary biliary cholangitis"},"content":{"rendered":"<div class=\"fpm_start\"><\/div>\n<p>Primary biliary cholangitis (PBC) is an autoimmune disease characterized by chronic inflammation and death of small bile ducts in the liver. This leads to cholestasis and subsequent liver damage, which can result in cirrhosis and liver failure. PBC is more common in women, especially in the age group of 40 to 60 years. Pathological changes in this disease develop slowly and can remain asymptomatic for many years, which complicates early diagnosis and timely treatment. The causes of the disease are not fully understood, but it is assumed that it is influenced by genetic, immunological and environmental factors.<\/p>\n<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_83 counter-flat ez-toc-counter ez-toc-light-blue ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Content<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" 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href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\" >History of the disease and interesting historical facts<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\" >Epidemiology<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\" >Genetic predisposition to this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Risk factors for the development of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Diagnosis of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\" >Treatment<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >List of medications used to treat this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Disease monitoring<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Age-related features of the disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/primary-biliary-cholangitis\/#%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\" >Questions and Answers<\/a><\/li><\/ul><\/nav><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\"><\/span>History of the disease and interesting historical facts<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Primary biliary cholangitis was first described in the mid-20th century, but although its existence had been noted earlier, a clinical understanding of the disease began to emerge only with the development of histological research methods. In the 1970s, the disease received the status of an independent clinical disease after the description of characteristic morphological changes in the liver. Since then, the term &quot;primary biliary cholangitis&quot; has become generally accepted. Interestingly, the disease was initially considered rare, but further studies have shown that its prevalence is much higher than expected. Modern medical research continues to focus on studying the pathogenesis of this disease, as well as on finding new effective treatments.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F\"><\/span>Epidemiology<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>According to the latest epidemiological study, the prevalence of primary biliary cholangitis is approximately 1-3 cases per 1000 people in all age groups, making it a fairly common disease in the population. It is important to note that the incidence varies significantly depending on the region, with higher rates observed in North American and European countries. PBC occurs predominantly in women, with a female to male ratio of approximately 9:1. Epidemiological data indicate a significant association with other autoimmune disorders, such as Sjogren&#039;s syndrome, scleroderma, and rheumatoid arthritis.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E\"><\/span>Genetic predisposition to this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Genetic predisposition plays a key role in the pathogenesis of primary biliary cholangitis. A number of studies suggest a link between the disease and certain genes associated with the immune response. First of all, the attention of researchers was focused on HLA genes, such as HLA-DR8 and HLA-DR3, which indicate an increased risk of PBC. Associations with changes in a number of genes responsible for the immune response, including genes associated with the regulation of T cells, were also identified. Some studies report the presence of mutations in genes regulating bile acid metabolism, which may also contribute to the development of the disease. However, the need for further genetic studies remains relevant for a deeper understanding of the mechanisms underlying this pathology.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Risk factors for the development of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Risk factors that contribute to the development of primary biliary cholangitis include both physical and chemical influences. Physical factors include:<\/p>\n<ul>\n<li>the presence of autoimmune diseases in the patient or his relatives;<\/li>\n<li>gender (female);<\/li>\n<li>age (women over 40 are most susceptible);<\/li>\n<\/ul>\n<p>Chemical risk factors include:<\/p>\n<ul>\n<li>exposure to certain toxic substances, such as chemicals commonly used in manufacturing;<\/li>\n<li>household and industrial chemicals containing carcinogenic substances;<\/li>\n<\/ul>\n<p>In addition, some infectious diseases (e.g. viral hepatitis) may also act as triggers for the development of PBC. Considerable attention has also been paid to the role of microbiota and intestinal dysbiosis, which highlights the importance of the environment in the pathogenesis of the disease.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Diagnosis of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Diagnosis of primary biliary cholangitis is a multi-stage process that includes clinical, laboratory and instrumental methods. <\/p>\n<p>The main symptoms to look out for are:<\/p><script data-noptimize=\"\" data-wpfc-render=\"false\">\nfpm_start( \"true\" );\n<\/script>\n\n<ul>\n<li>persistent itching of the skin;<\/li>\n<li>fatigue and weakness;<\/li>\n<li>dry eyes and mouth;<\/li>\n<li>pain in the right hypochondrium;<\/li>\n<li>changes in liver function (jaundice, enlarged liver).<\/li>\n<\/ul>\n<p>Laboratory tests include alkaline phosphatase and gamma-glutamyl transferase levels, which are often elevated in PBC. It is also important to test for mitochondrial antibodies (anti-M2) and assess immunoglobulin levels.<\/p>\n<p>Radiological studies such as ultrasound, MRI, and CT can help to exclude other pathologies of the biliary tract. A liver biopsy confirms the diagnosis by revealing characteristic inflammatory changes in the interlobes of the liver. Differential diagnostics are necessary to exclude other liver diseases such as primary sclerosing cholangitis and viral hepatitis.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\"><\/span>Treatment<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment of primary biliary cholangitis should be comprehensive and individualized. First of all, it is necessary to consider pharmacological methods. Most patients are prescribed ursodeoxycholic acid, which helps improve liver function and slows the progression of the disease. <\/p>\n<p>If the patient does not respond to drug therapy, surgical intervention may be recommended, including bile duct obstruction or liver transplantation if cirrhosis develops. <\/p>\n<p>There are other treatment approaches, such as the use of immunosuppressants such as methotrexate in combination with ursodeoxycholic acid. However, the effectiveness of these methods requires further research. It is also important to consider that lifestyle changes, including proper nutrition and vigorous physical activity, can have a positive effect on the course of the disease.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>List of medications used to treat this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The main drugs used to treat primary biliary cholangitis include:<\/p>\n<ul>\n<li>Urdosexic acid;<\/li>\n<li>Methotrexate;<\/li>\n<li>Sirolimus;<\/li>\n<li>Calcium and vitamin D;<\/li>\n<li>Immunosuppressants in severe cases.<\/li>\n<\/ul>\n<p>Each of these drugs may have its own indications and contraindications, which requires careful evaluation and monitoring by the physician.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Disease monitoring<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Monitoring of a patient with primary biliary cholangitis includes regular follow-up appointments to assess liver function, alkaline phosphatase levels, and other laboratory parameters. The prognosis of the disease is quite variable, but in the early stages and with adequate treatment, most patients can expect a satisfactory outcome. At the same time, it is important to be aware of the risks of complications such as primary sclerosing cholangitis and liver failure, which can seriously complicate the course of the disease.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Age-related features of the disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Although primary biliary cholangitis is most often diagnosed in women aged 40 to 60 years, it can also occur in adolescents and the elderly. In young people, the disease may progress more rapidly and manifest itself more acutely, while in older patients the course may be more latent and unexpressed. It is important that at each age, doctors take into account the individual characteristics of the course of the disease and accordingly adapt approaches to diagnosis and treatment.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\"><\/span>Questions and Answers<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li><strong>What are the main manifestations of primary biliary cholangitis?<\/strong> The main symptoms include itchy skin, fatigue, dry mouth and eyes, and pain in the right hypochondrium.<\/li>\n<li><strong>Is it possible to completely cure PBC?<\/strong> At present, a complete cure is impossible, but modern treatment can significantly improve the quality of life and slow the progression of the disease.<\/li>\n<li><strong>Does PBC affect life expectancy?<\/strong> With timely diagnosis and treatment, as well as active monitoring of the condition of many patients, it is possible to significantly prolong life and reduce the risk of complications.<\/li>\n<li><strong>What is ursodeoxycholic acid?<\/strong> Urdosexic acid is a drug that is used to improve liver function in patients with PBC and may slow the progression of the disease.<\/li>\n<li><strong>How often should follow-up examinations be performed?<\/strong> Follow-up examinations should be performed regularly, at least every six months, to assess liver function and the effectiveness of treatment.<\/li>\n<\/ul>\n<div class=\"fpm_end\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Primary biliary cholangitis (PBC) is an autoimmune disease characterized by chronic inflammation and death of the small bile ducts in the liver. This leads to<\/p>","protected":false},"author":1,"featured_media":21856,"comment_status":"open","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[298],"tags":[],"class_list":["post-12536","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-medlibrary"],"_links":{"self":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12536","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/comments?post=12536"}],"version-history":[{"count":1,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12536\/revisions"}],"predecessor-version":[{"id":14543,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12536\/revisions\/14543"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media\/21856"}],"wp:attachment":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media?parent=12536"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/categories?post=12536"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/tags?post=12536"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}